Blunted Hypercapnic Respiratory Drive Response in Subjects With Late-Onset Pompe Disease.
BACKGROUND: Patients with Iate-onset Pompe disease develop progressive hypercapnic respiratory failure that can be disproportionate to the respiratory muscle compromise and/or thoracic restriction. Although recent studies have reported the presence of a blunted hypercapnic respiratory response in so...
| Publicado en: | Respiratory Care Vol. 61; no. 7; pp. 930 - 936 |
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| Autores principales: | , , |
| Formato: | research tables/charts Journal Article |
| Publicado: |
Mary Ann Liebert, Inc.
Jul2016
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=116767827&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 116767827 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 00201324 4GG jtl: Respiratory Care issn: 00201324 maglogo: N pubinfo: dt: Jul2016 vid: 61 iid: 7 pid: 1365 pub: Mary Ann Liebert, Inc. place: New Rochelle, New York artinfo: ui: 116767827 116767827 116767827 10.4187/respcare.03940 116767827 ppf: 930 ppct: 6 formats: fmt: @attributes: type: P tig: atl: Blunted Hypercapnic Respiratory Drive Response in Subjects With Late-Onset Pompe Disease. aug: au: De Vito, Eduardo L. Monteiro, Sergio G. Aruj, Patricia K. affil: Instituto de Investigaciones Médicas Alfredo Lanari, Universidad de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina sug: subj: Glycogen Storage Disease Complications Carbon Dioxide Blood Respiratory Failure Physiopathology Respiratory Muscles Muscle Strength Evaluation Expiration Inspiration, Respiratory Airway Pressure Blood Gas Analysis Arteries Descriptive Statistics Data Analysis Software P-Value T-Tests Mann-Whitney U Test Fisher's Exact Test Case Control Studies Capnography Human Female Male Adult Middle Age Adult: 19-44 years Middle Aged: 45-64 years Female Male ab: BACKGROUND: Patients with Iate-onset Pompe disease develop progressive hypercapnic respiratory failure that can be disproportionate to the respiratory muscle compromise and/or thoracic restriction. Although recent studies have reported the presence of a blunted hypercapnic respiratory response in some subjects with neuromuscular disorders and chronic hypercapnia, no study has evaluated the integrity of the respiratory drive in subjects with late-onset Pompe disease. Thus, we endeavor to determine the CO2 rebreathing response in subjects with late-onset Pompe disease. METHODS: Respiratory muscle strength was assessed by measuring the maximum inspira tory pressure, and the maximum expiratory pressure. The maximum inspiratory pressure reflects the strength of the diaphragm and other inspiratory muscles, whereas the maximum expiratory pressure reflects the strength of the abdominal muscles and other expiratory muscles. We studied the hypercapnic drive response (measured as the ratio of the change in airway-occlusion pressure 0.1 s after the start of inspiration and end-tidal P... in 13 subjects with late-onset Pompe disease and 51 healthy controls. RESULTS: Overall inspiratory muscle strength was within normal limits or slightly diminished in the late-onset Pompe disease group. Five subjects (38.5%) were chronically hypercapnic, and 9 (69.2%) had an increased breath-holding time. Compared with controls, the change in airway-occlusion pressure 0.1 s/change in end-tidal CO2 pressure slope (hypercapnic respiratory drive) was lower in the late-onset Pompe disease group (median 0.050 [interquartile range 0.027-0.118] vs 0.183 [0.153-0.233], P < .001). Nine subjects (69.2%) had a blunted change in airway-occlusion pressure 0.1 s/change in end-tidal carbon dioxide pressure slope. CONCLUSIONS: Subjects with late-onset Pompe disease had an impaired hypercapnic respiratory drive response. The clinical impact of this phenomenon in this subject subset deserves further investigation. pubtype: Academic Journal doctype: research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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